Clinical manifestations in 28 Italian and Iranian patients with severe factor VII deficiency

Peyvandi, Flora; Pm, Mannucci; Asti, Daniela; Abdoullahi, M.; Rocco, Nicola; Sharifian, Roxana

doi:10.1046/j.1365-2516.1997.00137.x

Cited by 87 publications

(87 citation statements)

References 10 publications

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“…Some local studies of RBDs showed that FVII, FXIII and FX deficiencies are similar to what has been observed in our neighboring countries like India and Iran [13,14]. However in India, there is regional variation.…”

Section: Discussionsupporting

confidence: 74%

Rare Bleeding Disorders are not so Rare in Pakistan

Shamsi¹

2013

J Hematol Thrombo Dis

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Background: Consanguinity remains common in several populations around the world, and varies from country to country. In Pakistan, close consanguineous unions continue to be extremely common as in South West Asia. Here, we describe the frequency of Rare Bleeding Disorders (RBDs), their types and clinical features among patients seeking advice for bleeding tendencies from a single centre in Pakistan.

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Section: Discussionsupporting

confidence: 74%

Rare Bleeding Disorders are not so Rare in Pakistan

Shamsi¹

2013

J Hematol Thrombo Dis

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“…Intermediate values around 30-40% of normal were obtained using human placenta or human recombinant thromboplastin (h-thromboplastin) was used [158]. Those findings have been confirmed by other studies [159,160,161].…”

Section: Measurement Of Fvii By Using Different Thromboplastinssupporting

confidence: 58%

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Chaireti¹

2013

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“…FVII deficiency previously, considered extremely rare, is now considered to be the most common autosomal recessive clotting factor deficiency, with a prevalence of 0.5-1.0 per 10 6 in some populations [5], such Italian and Iranian. There have been no published reports of the prevalence of severe factor VII deficiency in the African-American population.…”

Section: Discussionmentioning

confidence: 99%

Lack of bleeding in patients with severe factor VII deficiency

et al. 2005

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Factor VII deficiency, although rare, is now recognized as the most common autosomal recessive inherited factor deficiency. It is usually considered to be associated with bleeding only in the severely affected subject and heterozygotes (>10%) are not considered at risk. The general recommendation for surgery is to achieve a FVII level in excess of 15% (0.15 1U/mL). We present three cases of severe factor VII deficiency, each of whom appeared hemostatically competent based on clinical history. Subject 1 is a 33 year-old African-American female with a baseline FVII of <1%, who had a fractured tibia requiring open reduction with internal fixation without any FVII replacement and subsequently underwent successful laparoscopic knee surgery with a factor VII level measured at 6%. Subject 2 is a 58 year-old African-American female with a factor VII level of 9% who underwent an elective left total hip replacement without any factor replacement and had no excessive bleeding, but who sustained a pulmonary embolism postoperatively. Subject 3 is a 19-year-old African-American male with a baseline FVII of 1% with a history of active participation in football without noticeable injury and who underwent an emergent appendectomy without bleeding. These three cases represent individuals with the severe form of FVII deficiency who did not exhibit excessive bleeding when challenged with surgical procedures. The clinical history would appear the most valuable tool in predicting the likelihood of bleeding in these patients, and we suggest that the presumption that all patients with severe FVII deficiency should receive replacement therapy before surgical procedures may not be valid in all cases. Am.

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Clinical manifestations in 28 Italian and Iranian patients with severe factor VII deficiency

Cited by 87 publications

References 10 publications

Rare Bleeding Disorders are not so Rare in Pakistan

Rare Bleeding Disorders are not so Rare in Pakistan

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Lack of bleeding in patients with severe factor VII deficiency

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